Single-center Longitudinal Study Research Articles

Predicting weight gain in patients with cystic fibrosis on triple combination modulator.

Cystic fibrosis (CF) is caused by CF transmembrane conductance regulator (CFTR) gene mutations producing dysfunctional CFTR proteins leading to progressive clinical disease. Elexacaftor-tezacaftor-ivacaftor (ETI) remarkably improves lung disease but is associated with substantial weight gain. We performed a single-center longitudinal study predicting 6-month weight gain after ETI initiation. We used linear mixed effects modeling (LME) to determine association of ETI treatment with changing body mass index (BMI). Using linear regression, we examined BMI prediction models with distinct combinations of main effects to identify a model useful for patient counseling. We used up to eight commonly observed clinical characteristics as input variables (age, sex, percent predicted FEV1 [FEV1%], F508del homozygous state, pancreatic sufficiency, HgbA1c, prior modulator use and prior year number of pulmonary exacerbations). We evaluated 154 patients (19-73 years old, 54% female, FEV1% = 19-121, 0-6 prior year pulmonary exacerbations). LME demonstrated an association between ETI use and weight increases. Exhaustive testing suggested a parsimonious linear regression model well-fitted to data that is potentially useful for counseling. The two variable model shows that on average, BMI decreases by 0.045 (95% Confidence Interval [CI] = -0.069 to -0.021, p < 0.001) for every year of age and increases by 0.322 (CI = 0.142 to 0.502, p = 0.001) for each additional prior year exacerbation at the time of ETI initiation. Young patients with many prior year pulmonary exacerbations likely have the largest 6 month weight gain after starting ETI.

Electrocardiographic proarrhythmic changes in pregnancy of women with CHD.

Pregnancy-related physiological adaptations result in increased heart rate as well as electrocardiographic changes such as a mean QTc prolongation of 27 ms. Pregnant women with CHD are at increased risk for cardiovascular complications. The aim of this study was to identify risk factors for abnormally prolonged QTc interval-a risk factor for ventricular arrhythmias-in pregnant women with CHD. Retrospective longitudinal single-centre study. Pre-pregnancy demographic and electrocardiographic risk factors for abnormal QTc duration during pregnancy of (a) > 460 ms and (b) >27 ms increase were analyzed. Eighty-three pregnancies in 63 women were included, of which three had documented arrhythmias. All five Modified World Health Organization Classification of Maternal Cardiovascular Risk (mWHO) classeswere represented, with 15 pregnancies (18.1%) in mWHO class I, 26 (31.3%) in mWHO II, 28 (33.7%) in mWHO II-III, 11 (13.3%) in mWHO III, and three pregnancies (3.6%) in mWHO class IV. Heart rate and QTc interval increased, while QRS duration and PR interval shortened during pregnancy. QTc duration of > 460 ms was associated with increased pre-pregnancy QTc interval, QRS duration, and weight, as well as body mass index. QTc increase of > 27 ms was associated with increased heart rate prior to pregnancy. No significant associations of electrocardiographic changes with mWHO class or CHD type were identified. Increased QTc in pregnant women with CHD was associated with being overweight or having higher heart rate, QRS, or QTc duration prior to pregnancy. These patients should be monitored closely for arrhythmias during pregnancy.

Prediction of the Individual Aortic Stenosis Progression Rate and its Association With Clinical Outcomes

BackgroundThe progression rate of aortic stenosis differs between patients, complicating clinical follow-up and management. ObjectivesThis study aimed to identify predictors associated with the progression rate of aortic stenosis. MethodsIn this retrospective longitudinal single-center cohort study, all patients with moderate aortic stenosis who presented between December 2011 and December 2022 and had echocardiograms available were included. The individual aortic stenosis progression rate was calculated based on aortic valve area (AVA) from at least 2 echocardiograms performed at least 6 months apart. Baseline factors associated with the progression rate of AVA were determined using linear mixed-effects models, and the association of progression rate with clinical outcomes was evaluated using Cox regression. ResultsThe study included 540 patients (median age 69 years and 38% female) with 2,937 echocardiograms (median 5 per patient). Patients had a linear progression with a median AVA decrease of 0.09 cm2/y and a median peak jet velocity increase of 0.17 m/s/y. Rapid progression was independently associated with all-cause mortality (HR: 1.77, 95% CI: 1.26-2.48) and aortic valve replacement (HR: 3.44, 95% CI: 2.55-4.64). Older age, greater left ventricular mass index, atrial fibrillation, and chronic kidney disease were associated with a faster decline of AVA. ConclusionsAVA decreases linearly in individual patients, and faster progression is independently associated with higher mortality. Routine clinical and echocardiographic variables accurately predict the individual progression rate and may aid clinicians in determining the optimal follow-up interval for patients with aortic stenosis.

Long-term bowel functional outcomes following anal sphincter-preserving surgery for upper and middle rectal cancer: a single-center longitudinal study.

Despite advances in neoadjuvant chemoradiotherapy and anal sphincter-preserving surgery for rectal cancer, bowel dysfunction is still unavoidable and negatively affects patients' quality of life. In this longitudinal study, we aimed to investigate the changes in bowel function with follow-up time and the effect of neoadjuvant chemoradiotherapy on bowel function following low anterior resection for rectal cancer. In this study, 171 patients with upper or middle rectal cancer who underwent low anterior resection between 2012 and 2018 were included. Bowel function was assessed longitudinally with Memorial Sloan Kettering Cancer Center Bowel Function Instrument and Wexner scores every 6 months after restoration of bowel continuity. Patients with at least 2 follow-up visits were included. Overall, 100 patients received neoadjuvant chemoradiotherapy. Urgency, soilage, and fecal incontinence were noted within 24 months in the patients treated with neoadjuvant chemoradiotherapy. After 2 years of follow-up, significant bowel dysfunction and fecal incontinence were observed in the neoadjuvant chemoradiotherapy group. Low tumor level and neoadjuvant chemoradiotherapy were associated with delayed bowel dysfunction. Neoadjuvant chemoradiotherapy in combination with low tumor level was significantly associated with delayed bowel dysfunction even after 2 years of follow-up. Therefore, careful selection and discussion with patients are paramount.

Esophageal adenocarcinoma heterogeneity in clinicopathology and prognosis: A single center longitudinal study of 146 cases over a 20-year period

Recent genomic studies suggest that esophageal adenocarcinoma (EAC) is not homogeneous and can be divided into true (tEAC) and probable (pEAC) groups. We compared clinicopathologic and prognostic features between the two groups of EAC. Based on endoscopic, radiologic, surgical, and pathologic reports, tumors with epicenters beyond 2 cm of the gastroesophageal junction (GEJ) were assigned to the tEAC group (N = 63), while epicenters within 2 cm of, but not crossing the GEJ, were allocated to the pEAC group (N = 83). All 146 consecutive patients were male (age: median 70 years, range: 51–88) and White-predominant (98.6 %). There was no significant difference in gastroesophageal reflux disease, obesity, comorbidity, and the prevalence of Barrett's esophagus, and cases diagnosed during endoscopic surveillance. However, compared to the pEAC group, the tEAC group had significantly more cases with hiatal hernia (P = 0.003); their tumors were significantly smaller in size (P = 0.007), more frequently with tubular/papillary adenocarcinoma (P = 0.001), had fewer cases with poorly cohesive carcinoma (P = 0.018), and demonstrated better prognosis in stage I disease (P = 0.012); 5-year overall survival (34.9 months) was significantly longer (versus 16.8 months in pEACs) (P = 0.043). Compared to the patients without resection, the patients treated with endoscopic or surgical resection showed significantly better outcomes, irrespective of stages. We concluded that EACs were heterogeneous with two distinct tEAC and pEAC groups in clinicopathology and prognosis; resection remained the better option for improved outcomes. Condensed abstractEsophageal adenocarcinoma can be divided into true or probable groups with distinct clinicopathology and better prognosis in the former than in the latter. we showed that resection remained the better option for improved outcomes.

Paraneoplastic Neurological Syndromes as Initial Presentation of Tumors: An Eight-Year Single-Center Experience

Background: Paraneoplastic Neurological Syndromes (PNS) comprise a diverse group of disorders propagated by immune-mediated effects of malignant tumors on neural tissue. Methods: A single-center longitudinal study was performed including consecutive adult patients treated at a tertiary academic hospital between 2015 and 2023 and diagnosed with PNS. PNS were ascertained using the 2004 and the revised 2021 PNS-Care diagnostic criteria. Results: Thirteen patients who fulfilled the 2004 definite PNS criteria were included. PNS comprise diverse neurological syndromes, with neuromuscular junction disorders (54%) and limbic encephalitis (31%) being predominant. PNS-related antibodies were detected in 85% of cases, including anti-AChR (n = 4), anti-P/Q-VGCC (n = 3), anti-Hu (n = 3), anti-Yo (n = 1), anti-Ma (n = 1), anti-titin (n = 1), anti-IgLON5 (n = 1), and anti-GAD65 (n = 1). Thymoma (31%), small-cell lung cancer (23%), and papillary thyroid carcinoma (18%) were the most frequent tumors. Imaging abnormalities were evident in 33% of cases. Early immunotherapy within 4-weeks from symptom onset was associated with favorable outcomes. At a mean follow-up of 2 ± 1 years, two patients with anti-Hu and anti-Yo antibodies died (18%). Four and three patients fulfilled the 2021 PNS-Care diagnostic criteria for definite and probable PNS, respectively. Conclusions: This study highlights the clinical heterogeneity of PNS, emphasizing the need for early suspicion and prompt treatment initiation for optimal outcomes.

Levator muscle avulsion and subsequent vaginal delivery: longitudinal 8-year follow-up.

The aim of this study is to assess the evolution of levator avulsion from 1 year to 8 years after first delivery in women with subsequent vaginal deliveries compared to women without subsequent vaginal deliveries. Further, we aim to assess whether women with full and partial avulsion 8 years after first delivery have larger levator hiatus area and more symptoms of pelvic organ prolapse compared to women with normal levator insertion. In this single center longitudinal study 195 initially primiparous women were included and underwent transperineal ultrasound 1 year and 8 years after first delivery. Muscle insertion was assessed by tomographic ultrasound imaging in the axial plane. Full levator avulsion was defined as abnormal muscle insertion in all three central slices. Partial levator avulsion was defined as abnormal muscle insertion in one but less than three central slices. Eight years after first delivery levator hiatus area was assessed at rest, during maximum pelvic floor muscle contraction, and maximum Valsalva maneuver. To assess symptoms of pelvic organ prolapse the vaginal symptoms module from the International Consultation on Incontinence Questionnaire was used. At 1 year follow-up, 25 women (12.8%) showed signs of avulsion, 20 women fulfilled sonographic criteria for full avulsion and 5 women fulfilled sonographic criteria for partial avulsion. Eight years after first delivery, 35 (17.9%) women were diagnosed with avulsion, of them 25 women were diagnosed with full avulsion and 10 women were diagnosed with partial avulsion. No women with partial and full avulsion at 1 year improved on their avulsion status at 8-year follow-up. In the subsequent vaginal delivery group, 21 women (14%) were diagnosed with partial and full levator avulsion 1 year after first delivery, and 31 women (20.7%) were diagnosed with partial and full avulsion 8 years after first delivery. Of the 45 women without subsequent vaginal delivery, one woman with partial avulsion 1 year after first delivery was diagnosed as full avulsion at 8-year follow-up. All women with full avulsion at 1-year follow-up were diagnosed as full avulsion at 8 years regardless of subsequent vaginal delivery. At 8-year follow-up women with full avulsion had statistically significant larger levator hiatus area compared to women with normal muscle insertion. Vaginal symptoms scores ranged between 5.5 (SD 5.7) and 6.0 (SD 4.0) and vaginal symptom quality of life scores ranged between 0.9 (SD 1.4) and 1.5 (SD 2.2) and did not differ statistically significantly between women with normal muscle insertion and women with partial and full avulsion at 8-year follow-up. More avulsions 8 years after first delivery compared to one year after first delivery were found in women with subsequent vaginal delivery. Except one primipara, none of the women without subsequent vaginal delivery changed on their levator status from 1 to 8 years after first delivery. Larger levator hiatus area was found in women with full avulsion compared to women with normal muscle insertion at 8-year follow-up. Vaginal symptoms scores were low and did not differ between women with normal muscle insertion, partial and full avulsion at 8-year follow-up. This article is protected by copyright. All rights reserved.

Significant improvements in clinical outcome measures and patient satisfaction after combined all-arthroscopic meniscal allograft transplantation and autologous chondrocyte implantation: A single-centre longitudinal study.

The optimal treatment approach for the complex pathology of meniscal insufficiency and coexisting full-thickness cartilage defects remains unclear. The purpose of this study was to evaluate the viability, safety, and efficacy of this combined surgical approach at medium-term follow-up. This is a single-centre longitudinal study with blinded outcome assessment. All consecutive patients treated with combined all-arthroscopic meniscal allograft transplantation (MAT) with bone bridge fixation and ACI using chondrospheres at our institution between 2001 and 2021 were eligible for inclusion. Twenty patients with an average follow-up of 72.6 ± 34.4 months were included in the statistical analysis. Clinical outcomes were assessed pre- and postoperatively using the IKDC Subjective Knee Form, Lysholm Score, Tegner Activity Scale, KOOS, and Visual Analog Scale (VAS) for patient satisfaction. Failure and reoperation rates were assessed, and cartilage regeneration tissue was evaluated on postoperative MRI. IKDC scores significantly improved from 52.1 ± 16.9 to 68.5 ± 16.3 (p = 0.003). Lysholm scores improved from 61.5 ± 21.7 to 78.5 ± 12.9 (p = 0.004). Tegner scores improved from 3.5 (1-4) to 4.0 (2-6) (p = 0.014). KOOS scores improved significantly across all subcategories, except 'symptoms', where improvements did not reach statistical significance. VAS for overall patient satisfaction showed improvements but did not reach statistical significance. The combined procedure was successful in 17 patients (85%). Eight patients had to undergo reoperation (40%), comprising mostly small, arthroscopic procedures. Seven reoperations were directly attributable to meniscal allograft transplantation (46.7%). Postoperative Magnetic Resonance Observation of Cartilage Repair Tissue scores were 68.9 ± 16.8 (n = 14). Combined arthroscopic MAT and autologous chondrocyte implantation (ACI) is a viable, safe, and effective treatment approach for younger patients with meniscal insufficiency and coexisting full-thickness cartilage damage, where alternative treatment options are limited. The combined surgical procedure achieved significant improvements in clinical outcome measures and patient satisfaction with acceptable failure and high arthroscopic reoperation rates. MAT is the limiting part of this combined procedure, with most failures and reoperations being attributable to MAT, as opposed to ACI. Level III.

[The use of the DOS and Delirium Prevalence: a quantitative longitudinal study at a Swiss-German central hospital].

The use of the DOS and Delirium Prevalence: a quantitative longitudinal study at a Swiss-German central hospital Abstract: Background: With a prevalence of 12-64%, delirium is a common complication in acute care, associated with negative outcomes such as increased mortality and prolonged length of stay. Many hospitals have guidelines to improve the delirium management. The Delirium Observation Screening Scale (DOS) Score is collected in the study hospital from all patients ≥ 70 years at each shift for at least 3 days. Delirium is diagnosed by a physician and coded according to ICD-10. Purpose: Evaluation of the delirium screening with the DOS according to internal guideline in terms of number of DOS assessments performed, prevalence of delirium (DOS score ≥ 3 points, CD-10 code delirium). Method: This retrospective quantitative single-centre longitudinal study used 2017 and 2018 data of 10046 cases. Statistical analysis methods were used to analyse prevalence of delirium and subgroup comparisons. Results: At least one DOS score was documented in 92% of cases aged ≥ 70-years (n = 5038). DOS implementation varied between 60% in the early, 49% in the late and 38% in the night shift. The prevalence of delirium was 12% according to DOS score ≥ 3 and 4% according to physician diagnosis of a delirium. Cases with a DOS score ≥ 3 were significantly older, more often female, had more comorbidities and were depressed. Conclusions: DOS is performed in most patients when indicated. The DOS implementation frequency varied depending on the shift.

A Multimodal Ayurveda and Mind-Body Therapeutic Intervention for Chronic Symptoms Attributed to a Postinfectious Syndrome: A Pilot Study.

Objective: Evaluate feasibility and impact of a multimodal integrative therapeutic intervention in patients presenting with chronic symptoms attributed to a postinfectious syndrome. Design: This was a prospective longitudinal single-center pilot study conducted from January 2019 to December 2020. Setting/Location: University of Maryland Lyme Program, Baltimore Maryland. Subjects: Persons presenting for Lyme evaluation for symptoms attributed to Lyme disease. Interventions: Participants attended two 1-h individual instructional sessions consisting of Ayurveda-based dietary intervention and breath-coordinated mind-body practice to be used for home practice. Outcome measures: Standard measures of impact were obtained at baseline, 1, 3, 6, and 12 months using the following validated survey instruments: Perceived Stress Scale (PSS), PROMIS Global Health v1.2 (GH), and PROMIS 29 v2.0 survey. Results: From 216 patients presenting for Lyme evaluation, 19 participants enrolled with 84% completing the study (N = 16). Baseline PROMIS GH scores consisting of general Physical Health (GPH) and general Mental Health (GMH) scores were lower in the study population than in the general U.S. population. PROMIS 29 scores were higher for fatigue, anxiety, and pain than those in the general U.S. population. Over 12-month period, improvement in both the GPH and GMH was 6.09 (confidence interval [95% CI] = 2.71-9.46; p < 0.001) and 4.65 (95% CI = 1.50-7.80; p = 0.004), respectively. PROMIS 29 scores showed the greatest improvement in fatigue at -7.91 (95% CI = -12.34 to -3.48; p < 0.001), pain interference -5.08 (95% CI = -9.20 to -0.96; p = 0.016), and ability to participate in social roles and activities 7.48 (95% CI = 3.21-11.75; p = 0.001) and least with depression -1.82 (95% CI = -4.74 to 1.10; p = 0.223). Employment status had significant effects on almost all outcome scores. Postinfectious state was associated with improvement in anxiety and PSS scores. Conclusions: A multimodal Ayurvedic and breath-coordinated mind-body therapeutic intervention is feasible and a potential nonpharmacologic therapeutic option for persons presenting with pain, stress, fatigue, physical dysfunction, and sleep disturbance attributed to a postinfectious syndrome. Further research is needed to determine efficacy in this population and in other groups with similar symptom complexes due to postinfectious syndromes.

Sexual function after radical cystectomy in males with bladder carcinoma: a six-year longitudinal single-centre study

IntroductionLittle is known about the long-term effects on sexual function (SF) after radical cystectomy (RC) in bladder carcinoma (BC) patients.AimTo assess the course of SF in BC patients who underwent RC, and individual characteristics that influence the sexual outcome during a six-year follow-up.MethodsIn this longitudinal study, 62 BC patients treated with RC were included between 2008 and 2022. Patients filled out validated questionnaires as part of the regular care at baseline, three months, six months, 12 months and thereafter yearly post RC.Outcome measuresTo determine the sexual function, the International Index of Erectile Function questionnaire was filled in and a linear mixed model for repeated measures was conducted. The analysed clinical variables included sexual preserving cystectomy (SPC), age, type of diversion, treatment, comorbidities, tumour status, smoking habits, patient status and open- or robot-assisted RC. A Generalised Linear Mixed Model was used to evaluate the impact on Quality of Life with the QLQ-C30 questionnaire.ResultsAfter an initial decrease of sexual function post-RC, no change in SF was seen during the six years of follow-up. A statistically significant difference in sexual health was found between SPC and standard RC (p=0.015), which was time-dependent. Patients with an orthotopic ileal neobladder experienced a significantly better SF than those with a Bricker’s ileal conduit (p=&amp;lt;0.001). A younger age also seemed to yield beneficial outcomes regarding SF (p=0.004). Other analysed clinical variables did not influence the course of SF. A statistically significant positive correlation was found between Quality of Life- and SF scores (p=0.004). Robot-assisted RC resulted in higher Global Health scores than open RC (p=0.001).ConclusionsRC has a severe impact on sexual function. Although SPC, collinear with the use of an orthotopic ileal neobladder and younger patient age show better outcomes in SF, erectile dysfunction post-RC is moderate to severe in the vast majority of patients.

Usability of Electronic Patient-Reported Outcome Measures for Older Patients With Cancer: Secondary Analysis of Data from an Observational Single Center Study.

Patient-reported outcomes are considered the gold standard for assessing subjective health status in oncology patients. Electronic assessment of patient-reported outcomes (ePRO) has become increasingly popular in recent years in both clinical trials and practice. However, there is limited evidence on how well older patients with cancer can complete ePRO assessments. We aimed to investigate how well adult patients with cancer of different age ranges could complete ePRO assessments at home and in a treatment facility and to identify factors associated with the ability to complete questionnaires electronically. This retrospective longitudinal single-center study involved survivors of cancer who participated in inpatient rehabilitation. Patients completed ePRO assessments before rehabilitation at home (T1) and after rehabilitation at the facility (T2). We analyzed the rate of patients who could complete the ePRO assessment at T1 and T2, the proportion of patients who required assistance, and the time it took patients to complete standardized questionnaires. Multivariate logistic regression analyses were conducted to identify predictors of ePRO completion rate and the need for assistance. Between 2017 and 2022, a total of 5571 patients were included in this study. Patients had a mean age of 60.3 (SD 12.2) years (range 18 to 93 years), and 1135 (20.3%) of them were classified as geriatric patients (>70 years). While more than 90% (5060/5571) of all patients completed the ePRO assessment, fewer patients in the age group of >70 years (924/1135, 81.4% at T1 vs 963/1135, 84.8% at T2) completed the assessment. Approximately 19% (1056/5571) of patients reported a need for assistance with the ePRO assessment at home, compared to 6.8% (304/4483) at the institution. Patients older than 70 years had a significantly higher need for assistance than those in younger age groups. Moreover, a gender difference was observed, with older women reporting a higher need for assistance than men (71-80 years: women requiring assistance 215/482, 44.6% vs men 96/350, 27.4%; P<.001 and >80 years: women 102/141, 72.3% vs men 57/112, 50.9%; P<.001). On average, patients needed 4.9 (SD 3.20) minutes to remotely complete a 30-item questionnaire (European Organization for the Research and Treatment of Cancer Quality of Life Questionnaire) and patients in the older age groups took significantly longer compared to younger age groups. Lower age and higher physical functioning were the clearest predictors for both the ePRO completion rate and the need for assistance in the multivariate regression analysis. This study's results indicate that ePRO assessment is feasible in older individuals with cancer, but older patients may require assistance (eg, from relatives) to complete home-based assessments. It may be more feasible to conduct assessments in-house in this population. Additionally, it is crucial to carefully consider which resources are necessary and available to support patients in using ePRO devices.

[Risk factors for cancer-related cognitive impairment in breast and colorectal cancer patients who undergo chemotherapy].

Our study aims to evaluate the impact of different factors on cancer-related cognitive impairment in patients who undergo chemotherapy. Prospective longitudinal single-centre study that included patients with breast and colon carcinoma who underwent chemotherapy as part of their treatment. Clinical and genetic characteristics of the patients (single nucleotide polymorphisms, SNPs) were collected. Patients' neurocognitive status was assessed using eleven validated tests at three time points: before chemotherapy (M0 - baseline), between one and four weeks after completing chemotherapy (M1), and between 24-30 weeks after completing chemotherapy (M2). Sixty-two patients were included in this study; 82% were female, median age was 56 years (range 30-74), and 64.5% had been diagnosed with breast cancer. Overall, better cognitive results at M0 were associated with age < 55 years, higher educational level, absence of comorbidities, and the CC variant rs471692 (TOP2A). Significant decline was found between M0 to M1 in the Rey Auditory Verbal Learning Test and the Letter and Number test, with evidence of recovery in M2 compared to M0 regarding the following test: Visual Memory, Functioning Assessment Short Test (FAST), Digit Symbol Substitution and Cube. In the multivariate analysis, being =55 years of age, adjuvant chemotherapy, presence of comorbidities, tobacco and alcohol use, and GT variant rs1800795 were associated with cognitive decline between M0 and M1. Being =55 years of age, female, presence of comorbidities and basic education level are related to a higher risk of cognitive impairment after chemotherapy.

Prediction of low-voltage areas in the left atrium in patients with non-valvular atrial fibrillation by non-invasive markers

Aim. To develop a method for predicting the area of low-voltage area (LVA) in the left atrium (LA), associated with the minimum and maximum expected effectiveness of primary radiofrequency ablation (RFA) in patients with non-valvular atrial fibrillation (AF) using non-invasive predictors.Methods. A longitudinal single-center study included 150 symptomatic non-valvular AF pts aged 20-72 years (median 59.0 [51.0; 64.0]), including 63 women (42%) hospitalized for primary RFA; 119 pts (79.3%) had paroxysmal and 31 (20.7%) - persistent AF. All pts initially underwent general clinical examination, transesophageal and advanced transthoracic echocardiography, estimation of NT-proBNP (pg/ml) and growth differentiation factor 15 (GDF-15, pg/ml) in the blood. Electroanatomical mapping was performed in sinus rhythm before RFA. The area of LVA (&lt;0.5 mV) was calculated as percentage of total LA area. Left ventricular (LV) ejection fraction (LVEF) was &gt;50% in all pts.Results. LVA area varied from 0 to 95.3%, median was 13.7% [5.1; 30.9]. Depending on LVA area, pts were divided into 3 groups: 36 pts (&lt;5%) in gr. 1; 74 pts (5-30%) in gr. 2; 40 pts (&gt;30%) in gr. 3. Increase of LVA area was associated with age, presence, and severity of congestive heart failure (CHF), persistent AF, CHA2DS2 -VASc score ≥3 points, increase of LA volume, LV hypertrophy and increase of NT-proBNP and GDF-15 levels. In univariate analysis, LVA area &lt;5% was associated with NT-proBNP level &lt;125 pg/ml, absence of obesity and CHF, lower LA volume index (&lt;28 ml/ m2). Independent predictors of LVA &lt;5% were: NT-proBNP &lt;125 pg/ml, absence of obesity and LA volume index ≤28 ml/ m2. The model was of good quality, C-statistics was 0.775 (p&lt;0.001). In univariate analysis, LVA area &gt;30 % was associated with age &gt;60 years, NT-proBNP &gt;125 pg/ml, GDF-15 &gt;840 pg/ml, persistent AF, presence of LV hypertrophy, LVEF ≤60%, LA volume index ≥ 32 ml/m2. Independent predictors of LVA &gt;30% were: LA volume index ≥32 ml/m2, GDF-15 &gt;840 pg/ml, and LVEF ≤60%. The model was of good quality, C-statistics was 0.752 (p&lt;0.001).Conclusion. Evaluation of noninvasive parameters, including clinical characteristics, echocardiographic parameters, and blood levels of NT-proBNP and GDF-15, allows prediction of electroanatomical substrate in left atrium in pts with non-valvular AF referred to primary RFA.

Histological healing as a predictor of sustained clinical remission in paediatric ulcerative colitis

BackgroundThe study aimed to assess the longitudinal impact of endoscopic healing (EH) and histological healing (HH) in a cohort of paediatric patients affected by ulcerative colitis (UC). MethodsThis was a retrospective single-centre longitudinal study. 86 children with UC who underwent endoscopic re-assessment while in clinical and biochemical remission were included. Partial EH was defined as a Mayo Endoscopic Subscore (MES) of 1 and complete EH was defined as a MES of 0. HH was defined as the absence of active inflammation in all biopsies. The cumulative incidence of clinical relapse was evaluated during follow-up. ResultsAt the second endoscopic re-evaluation, 59 (68.6%) patients achieved EH (MES ≤1). Of these patients, 39 (66%) achieved complete EH. 20 of the 39 patients who achieved complete EH attained complete HH. Patients who achieved partial and complete EH showed higher recurrence-free survival rates compared to those who did not (p < 0.01 and p < 0.01, respectively). Amongst patients with complete EH, those who achieved complete HH had lower recurrence rates when compared to patients who still showed microscopic inflammation (p = 0.049). ConclusionAchievement of EH and HH is associated with fewer disease relapses, with patients achieving HH showing longer relapse-free survival rates.

Assessment of chemotherapy-induced nausea and vomiting in pediatrics: Adequacy of control and adherence to guidelines

BackgroundChemotherapy-induced nausea and vomiting (CINV) is one of the most common side effects of chemotherapy in patients. Although sufficient evidence regarding its assessment and treatment in adults exists, CINV is more complicated in children. There are established guidelines for its prevention and treatment endorsed by several reputable organizations; however, their adherence varies between institutions. Therefore, this study aimed to assess the incidence and severity of CINV in pediatric patients receiving any form of chemotherapy in the acute and delayed phases and evaluated our institution's adherence to published guidelines by the Pediatric Oncology Group of Ontario. Material and methodThis was a prospective longitudinal single-center study. A structured assessment was administered twice to patients or their caregivers during the acute and delayed phases. Baxter Animated Retching Faces scale was used. ResultsA total of 186 patients completed 236 surveys, including those for acute and delayed phases. Incidence of nausea was reported in 33% patients in both phases, while vomiting was observed in 20.3% and 18.8% of the acute and delayed phases, respectively. A total of 31% patients met the criteria for proper adherence in case of prescribed appropriate antiemetic agent(s)/class irrespective of the dose. ConclusionCINV is a major adverse effect among children who receive high and moderate emetogenic chemotherapy compared to those receiving minimal and low emetogenic regimens. Despite the low rate of adherence to the guidelines, the incidence and severity of CINV reported in our study were consistent with previous studies. Further research should seek strategies to better implement and standardize the guidelines.

Impact of the Mean Cochlear Biologically Effective Dose on Hearing Preservation After Stereotactic Radiosurgery for Vestibular Schwannoma: A Retrospective Longitudinal Analysis.

Stereotactic radiosurgery (SRS) is a useful alternative for small- to medium-sized vestibular schwannoma. To evaluate whether biologically effective dose (BED Gy2.47 ), calculated for mean (BED Gy2.47 mean) and maximal (BED Gy2.47 max) cochlear dose, is relevant for hearing preservation. This is a retrospective longitudinal single-center study. Were analyzed 213 patients with useful baseline hearing. Risk of hearing decline was assessed for Gardner-Robertson classes and pure tone average (PTA) loss. The mean follow-up period was 39 months (median 36, 6-84). Hearing decline (Gardner-Robertson class) 3 years after SRS was associated with higher cochlear BED Gy2.47 mean (odds ratio [OR] 1.39, P = .009). Moreover, BED Gy2.47 mean was more relevant as compared with BED Gy2.47 max (OR 1.13, P = .04). Risk of PTA loss (continuous outcome, follow-up minus baseline) was significantly corelated with BED Gy2.47 mean at 24 (beta coefficient 1.55, P = .002) and 36 (beta coefficient 2.01, P = .004) months after SRS. Risk of PTA loss (>20 dB vs ≤) was associated with higher BED Gy2.47 mean at 6 (OR 1.36, P = .002), 12 (OR 1.36, P = .007), and 36 (OR 1.37, P = .02) months. Risk of hearing decline at 36 months for the BED Gy2.47 mean of 7-8, 10, and 12 Gy 2.47 was 28%, 57%, and 85%, respectively. Cochlear BED Gy2.47 mean is relevant for hearing decline after SRS and more relevant as compared with BED Gy2.47 max. Three years after SRS, this was sustained for all hearing decline evaluation modalities. Our data suggest the BED Gy2.47 mean cut-off of ≤8 Gy 2.47 for better hearing preservation rates .

Radiation exposure in pediatric patients with early onset scoliosis: A longitudinal single-center study

BackgroundThere is no consensus regarding the acceptable level of medical radiation exposure in patients with early-onset scoliosis. This study aimed to quantify radiation exposure in these patients and investigate factors associated with high exposure. MethodsPatients with early-onset scoliosis who received care for their spine deformity and other comorbidities in our institution were retrospectively reviewed. Cumulative radiation exposure and total number of imaging studies were recorded. Patients with ≥30 mSv exposure were classified as high exposure and analyzed to clarify factors associated with high exposure. ResultsThirty-five patients were included for analysis. The etiology of scoliosis was idiopathic in 8 patients, congenital in 7, syndromic in 8, and neuromuscular in 12. Fifteen patients underwent 19 spinal surgeries. The types of operation performed were definitive fusion (n = 12), vertebrectomy for hemivertebra (n = 2), growing rod (n = 1), lengthening (n = 3), and revision/partial implant removal (n = 1). The mean cumulative radiation dose was 22.3 mSv (range, 2.5–94.5 mSv). Spine radiography and computed tomography combined accounted for 15.0 mSv (range, 2.4–52.5 mSv, 67.3% of the mean cumulative dose). The mean radiation dose was significantly higher in patients who underwent spinal surgery than in those who did not (31.2 mSv vs. 15.6 mSv). The high-exposure group comprised 10 patients (1 idiopathic, 1 congenital, 5 syndromic, and 3 neuromuscular scoliosis) and 8 underwent 11 spinal operations. Among 8 patients who underwent spinal surgery, the cumulative radiation dose for spine was ≥30 mSv and spine computed tomography was performed an average of 4.0 times. ConclusionsNearly one-third of patients with early-onset scoliosis and half of patients who underwent spinal surgery had >30 mSv radiation exposure due to multiple computed tomography. Medical radiation exposure and associated cancer risk should be considered when treating these patients.

Neurosteroid pathway derangement in asphyctic infants treated with hypothermia: an untargeted metabolomic approach.

The pathobiological mechanisms associated with perinatal asphyxia and hypoxic-ischemic encephalopathy are complex and poorly understood. The metabolic effects of therapeutic hypothermia have been partially explored. We conducted a single-center longitudinal study to investigate the metabolic effects of perinatal asphyxia and hypoxic-ischemic encephalopathy on the urinary metabolome of a group of 12 asphyctic infants over time compared to 22 matched healthy newborns, using untargeted metabolomics based on mass spectrometry. Over-representation pathway analysis identified the steroidogenesis pathway as being significantly disrupted, with reduced steroid levels in the first three days of life despite treatment with hypothermia. Comparison with matched healthy newborns showed that the urinary steroid content was lower in asphyctic infants before hypothermia. The lysine degradation and carnitine synthesis pathways were also significantly affected. Steroidogenesis is significantly disrupted in asphyctic infants compared to healthy newborns. Given how neurosteroids are involved in neuromodulation and neuroprotection, translational research is warranted on the potential role of neurosteroid-based intervention in asphyctic infants. None.

Management of Retroperitoneal Fibrosis with Endovascular Aneurysm Repair in Patients Refractory to Medical Management

Early diagnosis and treatment of under-recognized retroperitoneal fibrosis (RPF) are essential before reaching the poorly responsive fibrotic stage. Although most patients respond to medical therapy, relapses are common. Open surgery in medically resistant patients is associated with major adverse clinical events. Therefore, we aim to compare the outcomes of endovascular repair in patients with RPF who were refractory to the optimal medical management. This is a single-center longitudinal study of optimal medical therapy (OMT) vs endovascular aneurysm repair (EVAR) in patients with RPF to our tertiary referral vascular center. Seventeen patients were referred to us with peri-aortic RPF over the study period. Eleven received OMT only, whereas six underwent EVAR after the failure of OMT. Eighty-two percent (n = 14) were male, and the mean follow-up was 74.40 ± 58.40 months. Nine (52%) had immunoglobulin G4-related disease (4 OMT vs 5 EVAR). EVAR patients had 100% technical success without perioperative mortality. Furthermore, all the EVAR patients were symptom-free following the intervention. Pre-operative aortic RPF index (maximum peri-aortic soft tissue diameter/maximum aortic diameter) was higher in the EVAR than in the OMT group. However, there was a significant decrement in the aortic RPF index following EVAR (P = .04). EVAR in RPF medical-resistant patients is a valuable option in the armamentarium of nephrologists and rheumatologists. Our study shows that an endovascular intervention provides a promising outcome in RPF/IgG4-RD periaortitis, which is refractory to medical therapy. We recommend further studies to investigate and establish the long-term effectiveness of endovascular repair in RPF.